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RAYNAUD'S PHENOMENON

Introduction

Raynaud's phenomenon (RP)and also known as Raynaud's syndrome or Secondary Raynaud's is less common than Raynaud’s Disease.  Raynaud's phenomena(RP)is typically associated withan underlying medical problem such as a connective tissue disease or autoimmune disorder.  Raynaud's phenomenon (RP)can also be associated with certain activitiesas in hand-arm vibration syndrome, with the use of certainmedications such as beta blockersforexample ergotamine, with occupational activities likevinyl chlorideexposureand can be aggravated by smoking.  Raynaud's phenomenon it is often a disabling problem and can affect patient's quality of life and Raynaud's phenomenon blood vessels can thicken with time and permanently reduced circulation to the affected areas if the artery becomes completely blocked there are increased chances for digital trophic changes chronic infections and even amputations.1-7

Pathophysiology

The cause of Raynaud's phenomenon (RP) is not fully understood. It is thought to be caused by hyperactivity of the sympathetic nervous system and local peripheral vascular changes.5

Incidence and Related Conditions

Raynaud's phenomenon is most commonly associated with scleroderma.  It is also present in 90% of the patients with mixed connective tissue disease; 15% of the patients with systemic lupus erythematosus (SLE) and 5% of the patients with rheumatoid arthritis.  It is also been seen in lymphoma, leukemia, frostbite, lead exposure, and diabetes.  It is associated with the use of some medications such as oral contraceptives and beta-blockers such as ergotamine.

Differential Diagnosis

  • Raynaud’s Disease
  • Carpal Tunnel Syndrome
  • Peripheral Vascular Disease
  • Reflex Sympathetic Dystrophy (Complex Regional Pain Syndrome)

Associated Diagnoses

  • Acrocyanosis
  • Cryoglobulinemia, mixed or type 3, associated with hepatitis B and C
  • Cryoglobulinemia, monoclonal or type I
  • Dermatomyositis
  • Fabry’s disease
  • Frostbite
  • Leukemia
  • Livedo reticularis
  • Lymphoma
  • Mycoplasma infection with cold agglutinins
  • Myeloma
  • Scleroderma, diffuse and localized (CREST syndrome)
  • Systemic lupus erythematosus 
  • Thromboangiitis obliterans
  • Thromboembolic disease
  • Thoracic outlet syndrome
  • Vasculitis
  • Vibration injury
  • Vinyl chloride exposure
  • Waldenström macroglobulinemia
ICD-10 Codes

  • RAYNAUD'S PHENOMENON

    Diagnostic Guide Name

    RAYNAUD'S PHENOMENON

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    RAYNAUD'S PHENOMENON w/o GANGRENEI73.00   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Raynaud Phenomenon
  • Raynaud phenomenon color changes in a patient with scleroderma.
    Raynaud phenomenon color changes in a patient with scleroderma.
  • Chronic hand and wrist changes in a patient with scleroderma
    Chronic hand and wrist changes in a patient with scleroderma
  • Chronic digital trophic changes secondary to scleroderma
    Chronic digital trophic changes secondary to scleroderma
  • Necrosis of long finger tip and previous amputation in chronic collagen vascular disease
    Necrosis of long finger tip and previous amputation in chronic collagen vascular disease
Symptoms
Chronic intermittent intense digital pain and ulcerations
Digital involvement is usually asymmetric
Significant functional handicaps
Swollen digits with marked loss of range of motion
Typical History

The patient is often a middle-aged female who describes the skin of her fingers or toes turning white, with feelings of cold and numbness, when exposed to cold. In addition, the patient has significant pain and marked disabling hand deformities.  She is disabled, distressed and depressed by her chronic loss of function.  She has chronic full thickness ulcers of both long fingers and the right index finger. She has seen several doctors and so far, the therapeutic interventions have either not helped and only provided very temporary relief.

Positive Tests, Exams or Signs
Work-up Options
Treatment Options
Treatment Goals
  • Establish an accurate disgnosis
  • Diagnosis associated underlying diseases
  • Maintain hand function
Conservative
  • Avoid rapidly changing temperatures
  • Quit smoking
  • Topical nitrates applications (2% nitroglycerin ointment)
  • Medications like calcium-channel blockers like nifedipine, alpha blockers, vasodilators)
  • Brachial plexus blocks
  • Sympathetic nerves blocks
  • Continuous regional anesthetic blocks
  • Botulinum Toxin Type A Injections into the palms
Operative
  • Thoracoscopic Cervicodorsal sympathectomy 5,7
  • Peripheral digital sympathectomies using microsurgical techniques 5,7
  • Vein grafting of arterial dysfunctional segments5,7
Complications
  • Nerve damage
  • Non-healing ulcers
  • Digital amputations
  • Chronic infections
Outcomes
  • Sympathectomy may reduce frequency and duration of attacks, but it is not always successful. 
  • Botulinum toxin A (Btx-A) around the common digital neurovascular bundles usually provides some pain relief and ulcer healing.  However, Botulinum toxin A (Btx-A) should be avoided in patients with neuromuscular diseases such as myasthenia gravis.
  • Peripheral digital sympathectomies usually improves circulation, decreases cold intolerance and heals ulcers.5-7
Key Educational Points
  • Raynaud's phenomenon is intermittent digital artery vasospasm stimulated by cold and stress. Patients experience transient blanching followed by cyanosis, hyperemia and pain.
  • Laboratory findings in scleroderma include a positive ANA test with a nucleolar pattern and sometimes a positive rheumatoid factor.
  • Patients with Raynaud's Disease typically are healthy females under age 20 at onset, have mild vasospastic attacks caused by cold or emotional stress, the color changes are symmetrical in both hands and they have no necrosis or gangrene.10
  • Patients with Raynaud's Phenomenon typically have onset of symptoms around age 30, the vasospastic attacks are intensely painful, asymmetric in the hands and are associated ischemic skin changes. The patients also demonstrate signs suggestive of a connective tissue disease like scleroderma or rheumatoid arthritis.10
  • The triphasic color changes of Raynaud’s phenomenon or disease; pale (white), cyanotic (blue) and reperfusion hyperemia (red) are a key characteristic of Raynaud's disease and Raynaud's phenomenon.
References

New Articles

  1. Nietert PJ, Shaftman SR, Silver RM, et al. Raynaud phenomenon and mortality: 20+ years of follow-up of the Charleston Heart Study cohort. Clin Epidemiol2015;7:161–8. PMID: 25678814
  2. Chatterjee S. When do Raynaud symptoms merit a workup for autoimmune rheumatic disease? One-Minute Consult. Cleveland Clin J Med2013;80(1):22-5. PMID: 23288940
  3. Carpentier PH, Satger B, Poensin D, Maricq HR. Incidence and natural history of Raynaud phenomenon: a long-term follow-up (14 years) of a random sample from the general population. J Vasc Surg2006;44(5):1023-8.PMID: 17098538
  4. Hodges H. Raynaud's disease: pathophysiology, diagnosis, and treatment. J Am Acad Nurse Pract1995;7(4):159-64. PMID: 7756043
  5. Porter SB, Murray PM.  Raynaud phenomenon. J Hand Surg Am. 2013; 38A:375-378.
  6. Butendieck RR, Murray PM.  Raynaud disease.  J Hand Surg Am. 2014; 39A:121-124.
  7. Neumeister MW.  Botulinum toxin type A in the treatment of Raynaud’s phenomenon. J Hand Surg Am. 2010; 35A:2085-2092.
  8. Wigley FM. Raynaud’s phenomenon. N Engl J Med 2002; 347(13); 1001-1008.

Reviews

  1. Mayo Clinic. Raynaud’s disease. 2016. Accessed October 28, 2016 at http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/definition/con-20022916

Classics

  1. Jepson RP. Raynaud phenomenon. Postgrad Med J.1953 Sep;29(335):451-4. PMID: 13088515
  2. Birnstingl M. The Raynaud syndrome. Postgrad Med J1971;47(547):297-310. PMID: 4931707
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